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  • 1
    Electronic Resource
    Electronic Resource
    The Malmö model for immune tolerance induction: impact of previous treatment on outcome (2000)
    Oxford UK : Blackwell Science Ltd
    Haemophilia 6 (2000), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Ten patients, who had been treated according to the Malmö model for immune tolerance induction (ITI), were analysed regarding treatment with clotting factors and chemotherapy during the time period between inhibitor detection and ITI. Of the patients who were successfully rendered tolerant (n=6) all but one had received treatment with FVIII, either alone (n=1), or combined with cyclophosphamide (n=4). Of the patients who did not become tolerant, three of four had received treatment with FVIII during the inhibitor period but only one with FVIII and chemotherapy. The total amount of treatment received was in general much lower in the group that did not become tolerant. In individual cases, it appeared very clear that the inhibitor level and anamnestic response was substantially reduced prior to the ITI using the Malmö treatment model. We conclude that treatment of acute bleeds during the inhibitor period may be of importance for ITI and that the different response rates published for different immune tolerance regimens most likely do not reflect the true response rate for the respective regimen.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2000.00436.x
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    On-demand vs. prophylactic treatment for severe haemophilia in Norway and Sweden: differences in treatment characteristics and outcome (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  Using an 11-year panel of 156 Norwegian and Swedish patients with severe haemophilia, and including retrospective case-book data from birth, we compared the differences in the haemophilia-related resource use between on-demand and prophylactic treatment. Patients treated on-demand had more surgery (arthrodeses, prostheses implantations and synovectomies) and more days lost from work. Median annual factor-concentrate consumption among adults (18+) was 211 000 IU [interquartile range (IQR) 154 000–268 000] or 3 024 IU kg−1 year−1 for patients on prophylactic treatment and 55 000 IU (IQR 28 000–91 000) for on-demand patients (780 IU kg−1 year−1). This was partly explained by the fact that the median dose per kg body weight was twice as great 28, (IQR 24–32) for prophylaxis compared with 14 (IQR 12–16) for on-demand. Prescribed dose per kg body weight was found to be an important factor explaining the variation in total annual factor-concentrate consumption per patient for both types of treatment. Other variables included in the panel-data regression analysis were the number of weeks on secondary prophylaxis for on-demand patients and age, body weight and type of haemophilia for children (0–17 years) on prophylaxis. Differences were consistently substantial and will affect both costs and benefits of the two treatment strategies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00817.x
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Economic evaluation: what are we looking for and how do we get there? (2004)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 10 (2004), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  The interest in economic evaluation of alternative strategies for haemophilia treatment has increased through the years. Few studies have actually been undertaken, however, and most of them have been simple cost-minimization or cost-effectiveness analyses. From the perspective of the binational project ‘Treatment strategies for severe haemophilia − prophylaxis vs. on-demand’, the present paper discusses the pros and cons of different methods for economic evaluation and their data requirements. Severe haemophilia is a rare disease that requires lifelong treatment. In addition, treatment has both short- and long-term effects which are likely to differ between strategies. Accordingly, regardless of the chosen evaluation method, data requirements are non-trivial. Hence, the various problems connected to the generation of data, as well as how they may be addressed, are also discussed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1355-0691.2004.00878.x
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Experience with a new percutaneous port system, Percuseal®, for intravenous injection in patients with haemophilia, von Willebrand disease and severe alpha1-antitrypsin deficiency (2003)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 9 (2003), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  We have implanted a new port system (Percuseal®) in altogether 13 patients with haemophilia A, B, von Willebrand disease and alpha1-antitrypsin deficiency in order to facilitate venous access. The Percuseal system differs from subcutaneous ports, such as Port-a-Cath, in that the upper part of the device protrudes above the skin. In this way, the patient can easily puncture the port membrane under the guidance of his eyes without penetrating the skin. In the present study cohort, a number of complications occurred. These were mainly caused by repeated local infections (in five patients), which made it necessary to replace the ports in three of the patients and to permanently remove the ports as the first option in two of the patients. In one patient, the port was removed because of inconvenience when doing physical exercise. In one additional patient, a severe systemic infection occurred, causing spondylitis. Despite the high infection rate, most patients considered the device very convenient to use. Because of the side-effects seen in our study, the Percuseal port in its present form is not to be recommended for regular use. A reconstruction of the port, making it smaller and giving it an antibacteriostatic cap, may possibly make this kind of port system a feasible alternative to use in order to improve pharmacoeconomics in the prophylactic treatment of haemophilia and patients with alpha1-antitrypsin deficiency.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.2003.00724.x
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    HIV-disease progression in Swedish haemophiliacs and the influence of replacement therapy (1997)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 3 (1997), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: HIV-disease progression in terms of the decline in CD4+ cell count, the development of AIDS-related symptoms and death was studied in 100 Swedish HIV-positive haemophiliacs and correlated to age and haemophilia treatment.On average 15 years after seroconversion, 66% of the patients had CD4+ cell counts of 〈 200×106 L−1, 48% had developed AIDS and 56% had died. Age was found to correlate to all three endpoints, also after adjustment for age, annual clotting factor concentrate (CFC) consumption and HIV-related therapy, i.e. pneumocystis prophylaxis and antiretroviral drugs (P 〈 0.05). Total annual CFC consumption showed no significant relationship to the decline in CD4+ cell counts but was inversely correlated to both the development of AIDS-related symptoms (P = 0.033) and mortality (P = 0.014). Prophylactic treatment was not associated with significantly better survival than on-demand treatment after adjustment for age, CFC consumption and HIV-therapy. The use of monoclonal-antibody-purified CFCs was not found to stabilize the decline in CD4+ cell counts. However, the use of these CFCs was inversely correlated both to the development of AIDS-related symptoms and to mortality (P = 0.042 and 0.027, respectively). A similar trend was associated with the use of low- and intermediate-purity CFCs. As compared with the severe haemophilia A subgroup, the moderate haemophilia A patients showed a trend toward slower disease progression, possibly attributable to a lower incidence of haemarthrosis and arthropathy among the latter.We conclude that replacement therapy in HIV-infected haemophiliacs is important also for HIV-disease progression, whereas the purity of the CFCs and the regimen used are of minor importance.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1997.00122.x
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    Paper (German National Licenses)
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  • 6
    Electronic Resource
    Electronic Resource
    Classification of von Willebrand disease: emerging issues (1999)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 5 (1999), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Among the three principal types of vWD, type 2 includes all qualitative abnormalities of vWF, and type 3 is the recessive form with total or near total absence. Type 1 with a purely quantitative reduction of vWF is considered to be the most common. As opposed to types 2 and 3, the molecular genetic background of type 1 has not been detected except in a few cases. We found that almost 25% of our patients diagnosed as type 1 vWD several years ago had the substitutions R611C och R552C recently labelled as type 2 mutations based on in vitro and/or clinical studies. The laboratory characteristics and the good response to DDVP, however, make them difficult to distinguish from type 1. Obviously, these substitutions confer only a minor and clinically insignificant functional deficiency of the vWF when present in heterozygous form. Based on this experience we question the clinical usefulness of the present vWD classification.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1999.0050s2046.x
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    Paper (German National Licenses)
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  • 7
    Electronic Resource
    Electronic Resource
    Haemostatic treatment in connection with surgery in patients with von Willebrand disease (1999)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 5 (1999), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Haemostatic treatment in patients with von Willebrand disease (vWD) in connection with surgery aims at normalizing the haemostatic defect in order to avoid bleeding complications. Factor VIII (FVIII) levels in plasma must be normalized in connection with major surgery, whereas the bleeding time is more important for mucous membrane bleedings. Most patients respond well to treatment with desmopressin which stimulates the endogenous release of FVIII and von Willebrand factor (vWF) and shortens the bleeding time. Non-responders to desmopressin are substituted with a plasma-derived factor concentrate which contains vWF and FVIII. This paper includes a summary of retrospective data from the last 10 years on haemostatic treatment in connection with surgery from four haemophilia centres in Sweden and Denmark on 40 invasive procedures in 27 vWD patients and on one normal delivery. If a FVIII-containing concentrate is given prior to surgery a dose of 30–40 IU VIII:C kg−1 will normalize FVIII levels in most severe cases. If a pure vWF concentrate is used, a dose of 40–50 IU RCoF kg−1 will normalize RCoF in most cases, but FVIII levels will not be normalized until after about 12 h or later. Repeated doses of FVIII-vWF concentrate may lead to very high levels of FVIII in plasma because of the combined effect of the exogenous FVIII-substitution and the endogenous FVIII-release induces by the infused vWF. Dosage should be adjusted according to FVIII levels in plasma.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1046/j.1365-2516.1999.0050s2064.x
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    Paper (German National Licenses)
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  • 8
    Electronic Resource
    Electronic Resource
    Costs of on-demand and prophylactic treatment for severe haemophilia in Norway and Sweden (2004)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 10 (2004), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  The expected annual cost (in the year 2000 prices) for a 30-year-old patient with average individual and treatment characteristics for on-demand EUR 51 832 (95% CI: 44 324–59 341) and for prophylaxis EUR 146 118 (95% CI: 129 965–162 271), was obtained from panel-data analysis of an 11-year retrospective panel of 156 patients with severe haemophilia in Norway and Sweden. Costs included haemophilia-related treatment costs within the health-care sector (factor concentrate, doctors’ visits, diagnostic procedures, hospitalisation, invasive procedures, etc.) and cost for haemophilia-related resource use in other sectors (lost production, use of special equipment, adaptation of workplace and domicile, etc). Although costs of lost production, reconstructive surgery and hospitalisation were higher for on-demand, they did not balance out the higher costs of factor-concentrate consumption in prophylaxis. The cut-off risk of premature death, where on-demand and prophylaxis would have been equally costly, was 3.7 percentage units higher for on-demand than for prophylaxis. Such a great risk difference has not been reported elsewhere to our knowledge. Estimated cost-elasticities indicated that annual costs of prophylaxis would increase by approximately the same proportion as a potential increase in the price of factor concentrate and decrease less than proportionately with a reduction in prescribed dose kg−1. For on-demand, the annual costs would increase by approximately the same proportion as an increase in the prescribed dose kg−1.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.00952.x
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    Paper (German National Licenses)
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  • 9
    Electronic Resource
    Electronic Resource
    The administration of desmopressin by nasal spray: a dose-determination study in patients with mild haemophilia A or von Willebrand's disease (1995)
    Oxford, UK : Blackwell Publishing Ltd
    Haemophilia 1 (1995), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Desmopressin is an important haemostatic agent in many types of bleeding disorders. The intranasal route for administration of desmopressin has attracted much interest because it is convenient and makes self-treatment possible. In the present study the effects of three doses (300, 450 and 600 μg) of desmopressin delivered by nasal spray and those of 0.3 μg/kg injected intravenously were compared in five patients with haemophilia A and 11 with von Willebrand's disease. There was no statistically significant difference in peak VIII:C concentration in the haemophilia patients and no difference in peak vWF concentration in von Willebrand patients between the four desmopressin dosages. Bleeding time response was comparable after all four dosages. We recommend a spray dosage of 300 μg for home treatment. As cover for major surgery or in connection with severe bleedings, however, intravenous administration is to be recommended.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.1995.tb00047.x
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    Paper (German National Licenses)
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  • 10
    Electronic Resource
    Electronic Resource
    Willingness to pay for on-demand and prophylactic treatment for severe haemophilia in Sweden (2004)
    Oxford, UK : Blackwell Science Ltd
    Haemophilia 10 (2004), S. 0 
    Details
    ISSN: 1365-2516
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary.  The objective of the present paper was to provide an estimate of the benefits of on-demand and prophylaxis treatment strategies for severe haemophilia in monetary terms. Using the contingent-valuation method, which simulates a missing market by asking people about their willingness to pay (WTP), we asked a representative sample (n = 609) of the Swedish population if they would be willing to pay a specific amount (bid) so that patients with severe haemophilia could receive on-demand treatment and another bid for prophylactic treatment. Different respondents were offered different bids and the bid vector ranged from 71 Euro cents to EUR 130. The order of the bid questions was randomized so that half of the respondents were asked first about their WTP for on-demand treatment, and then about their WTP for prophylaxis, while the order was reversed for the other half of the respondents. The mean estimated WTP (year 2002) was EUR 39 (95% CI 31–47) for on-demand and EUR 65 (95% CI 55–73) for prophylaxis. Our sensitivity analysis showed that the ranking of the two treatment alternatives was robust in that the WTP was greater for prophylaxis in all possible subsets. The point estimates of WTP varied somewhat in subsets defined by individual characteristics, but confidence intervals always overlapped that of the main results. The WTP for on-demand and prophylaxis exceeded the calculated cost of treatment per taxpayer of providing on-demand and prophylactic treatment, respectively, based on our previous results [1].
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1111/j.1365-2516.2004.00954.x
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